Silence of sickle cell in children doesn’t always indicate mild disease

Sickle cell disease (SCD) is a dynamic condition that affects people differently. Its symptoms and complications are evident in some patients from childhood, while in others, it may be silent or asymptomatic.

There is a common misconception that every child who lacks obvious symptoms or has an “asymptomatic” phase has a mild form of the disease. However, this is not always the case. While some patients do experience mild disease during childhood and adulthood without being on any disease-modifying therapies, for others, a childhood dormancy period is not equivalent to a mild form of SCD; rather, it’s the calm before the storm.

This phenomenon can be likened to a Yoruba proverb: “Agbo to tadi meyin, agbara lo lo mu wa,” which means that the retreat of a ram trying to attack you doesn’t necessarily stem from fear; it may be gathering momentum to launch a more powerful attack. Similarly, the silence of SCD doesn’t mean the disease isn’t causing damage. It could be silently progressing, accumulating harm that will manifest later.

Therefore, if your child with SCD appears well, it’s crucial not to become complacent. This period of seeming normalcy shouldn’t lead you to abandon prescribed daily medications, skip clinic appointments, or disregard your doctor’s instructions. Do not assume that your child’s stable health is solely due to herbal concoctions, one healthy meal you cooked, or a supplement your friends recommended that magically cured SCD. The underlying disease process remains.

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As a physician, I’ve encountered many people who were unaware of their SCD diagnosis until their teenage years or even their late 20s, and therefore received no treatment until much later in life when they were alarmed by a severe complication. Some parents, perhaps out of a desire to shield their children or due to the initial mildness of the disease, withheld the diagnosis. But their children went on to experience a relentless barrage of complications in their late teens or in their 20s, forcing them to confront their condition.

I can personally relate to this. While I was growing up, most people didn’t know I had SCD, and those who did assumed my case was mild because I had only occasional pain crises. I wasn’t hospitalized until I was 11 years old, and I didn’t receive a blood transfusion until my early 20s. It wasn’t until my late teens that SCD began to relentlessly bombard me with a series of health challenges.

The reality is that SCD is progressive, and it can take time for the damage it causes to become evident. Sometimes this damage occurs in childhood, but the effects aren’t yet significant enough to result in outward symptoms or signs. Instead, it may not manifest until adulthood, causing significant complications that may not be reversible — especially if necessary precautions to delay progression or reduce severity have been ignored. This is why adult patients experience more complications than children.

As a proactive caregiver, do not be misled by a child’s asymptomatic period or the apparent mildness of their disease. It’s vital to provide adequate care and always adhere to specialists’ guidelines. Never assume your child is fine and doesn’t need to attend appointments simply because they are asymptomatic.

Individuals who truly have a mild form of the disease are expected to consistently experience mild symptoms throughout their lives and not encounter extremely serious complications at any point. Those who begin experiencing severe complications in their late teens or 20s never had a mild form of the disease; they simply assumed so because it was initially silent.

Parents, never take your child’s health for granted. The way you manage their health today can significantly impact their well-being in the future.

I believe it is crucial for every patient living with SCD to undergo advanced hemoglobin electrophoresis tests to quantify all of the hemoglobin in their red blood cells. The basic hemoglobin electrophoresis test usually performed here in sub-Saharan Africa doesn’t have this capability. Aside from genotype, the advanced tests can provide valuable insights into whether your child possesses factors associated with a mild disease, such as fetal hemoglobin greater than 20% or the coexistence of alpha thalassemia with SCD. Although not all severity determinants can be quantified, it is beneficial to know the ones that can.

To reiterate, don’t let the silence of SCD in children keep you from taking necessary precautions.

Note: Sickle Cell Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Sickle Cell Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to sickle cell disease.